Phenotype and Long-Term Outcome in Recurrent Paediatric Acute Liver Failure: Systematic Review and Individual Participant Data Analysis.

Summary

This systematic review with individual participant data analysis examines recurrent paediatric acute liver failure associated with NBAS, RINT1, LARS1 and SCYL1 mutations. It pools 168 children from case reports, series and cohorts, describing age at presentation, frequency of episodes, association with febrile illness, and rates of liver transplantation and death. The authors conclude most cases present in infancy with a largely self-limiting course, and suggest genetic testing in unexplained acute liver failure with fever. Useful background for PICU teams managing children admitted with acute liver failure.

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